Yvonne S. Sininger PhD Professor Emeritus, UCLA

Consultant, C&Y Consultants, Santa Fe, NM

ysininger@cnyconsult.com

Newborn Hearing Screening: Current Best Practice and

Potential Improvements

1

Joint Committee Statement 2000

All infants have access to hearing screening using a physiologic

measure during their hospital birth admission. (UNHS) (ONE)

All infants who do not pass the screening begin appropriate audiologic

and medical evaluations to confirm the presence of hearing loss before

3 months of age. (THREE)

All infants with confirmed permanent hearing loss receive services

before 6 months of age. (SIX)

2

Screening leads to Earlier ID and Intervention

•UCLA Study/ Sininger Auditory

Development in Early-Amplified Children

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Age Dg

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Age Dg

Data from prospective study of

62 children with hearing loss,

16 not screened, 46 screened.

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e in

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Screened

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Screened Screened

SININGER, et al., (2009), Journal of American Academy of Audiology 20:49-57

3

Intervention before 6 Months Leads to Near-Normal Language Skills Yoshinaga-Itano et al.

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10

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18 Mo 24 Mo 30 Mo 36 Mo

ID before 6 Mo.

ID after 6 Mo. Language

Age in

Months

Actual Age in Months

Auditory Development in Early Amplified Children

Pediatric Speech Intelligibility

IMSPAC (Imitative Test of Speech Pattern Contrast Perception-On Line)

Predictive Measures:

• Age at Amplification

• Degree of Hearing Loss

• Cochlear Implant Status

• Intensity of Intervention

• Parent/Child Interaction-NCAST

• Multi-lingual Home

Outcome Measures:

Speech Perception

Speech Production

Language

Arizona 3

Reynell Language

Expressive & Receptive

SININGER, Y., GRIMES, A., CHRISTENSEN, E., (2010) Auditory

Development in Early Amplified Children: Factors Influencing

Auditory-Based Communication Outcomes in Children with Hearing

Loss. Ear and Hearing 31(2): 166-85.

5

Multivariate Least Squares Regression Analysis

Age at Amplification

Degree of Loss

Cochlear Implant

Intensity of Intervention

Parent/Child Interaction

Multi-lingual Home

Factors

Speech Production

Spoken Language

Expressive

Receptive

Speech Perception in Noise

Speech Feature Perception

Outcomes

Used to

Model

6

The factor that is most important in predicting overall outcomes: Age at Fitting of Hearing Aids

Each month delay in fitting is associated with:

• 3/4 month delay in Speech Feature Perception

• 3/4 months delay in Speech in Noise Perception

• .02 Z Score points decrease in Speech Production

• 1/3 months delay in Expressive Language

• .2 months delay in Receptive Language

7

2) Hearing Level

Each 10 dB of additional loss is associated with:

• Loss of .3 Z-score points on Speech Production

• 5.2 months lag in Expressive Language

• 5.9 months lag in Receptive Language

8

NB Hearing Screening

in the US

Where do we stand? 9

2013 JCIH POSITION STATEMENT UPDATE TO 2007

PEDIATRICS Volume 131, Number 4, April 1, 2013

Principles and Guidelines for Early

Intervention After Confirmation That a

Child Is Deaf or Hard of Hearing

10

EHDI in the United States

• Every state has it’s own laws and guidelines regarding

screening but all are based on JCIH Principles

• 98 or 99 percent of all children born in the United States

are screened for hearing loss by age 1 month.

• NICU infants generally are screened with ABR but

otherwise there is no directive as to method of screening

11

Technology & Protocols

• No standardized protocols exist except that ABR is

recommended for high risk infants to detect AN.

• Well baby nurseries use ABR/ASSR or OAE (transient or

dpoae) or a combination of both.

• A common strategy is to screen with OAE and retest, if

necessary with ABR.

12

California Testing Protocol No specific technology or equipment.

All infants screened before discharge.

Hospital pass rate standards: >90% for OAE >95% for ABR

Two (NICU) or three screens before diagnostic hearing test.

Diagnostic hearing test before 3 months

Intervention before 6 months

Sacramento

San

Francisco

Los

Angeles

All Hospitals must be trained and

certified.

Hospitals MUST make a follow up

appointment for all failed screens!

Hospital reports all findings to parents

and to primary care physicians.

All fails and follow-up times are sent to

the local HCC.

HCC monitors results of follow-up as

well as no-shows.

Dx centers and HCC work on

contacting families.

Features of California’s NHS Program

14

Mandated in all birthing Hospitals

$30 payment for uninsured or

Medicaid.

Standards for hearing health services.

Statewide infant tracking

Geographically-based Hearing

Coordination Centers

5% Loss to Follow-up due to State-wide

tracking system

Features of California’s NHS Program

15

Follow-up

materials in >25

languages!

16

DX

Test

Method

A prospective study was carried out on 1405 neonates (983 well born babies and 422 high risk babies) who were screened

during May 2013 to January 2015 at Institute of Obstetrics and Gynecology, Madras Medical College, Chennai. All neonates

were screened using two step screening protocol. They were initially tested with DPOAE. Referred babies in DPOAE were

screened with AABR subsequently.

Results

Among 1405 (100%) neonates 983 (69.96%) were well born babies and 422 (30.03%) were high risk babies. Total referral rate

in DPOAE was found to be 311 (22.13%) among which 195 (13.87%) were well born babies and 116 (8.25%) were high risk

babies. Out of 311 babies 31 (2.20%) babies were referred in AABR screening. In 31 babies referred in AABR 11(0.78%)

were from well born group and 20 (1.42%) were from the high risk group. Further diagnostic evaluation of these babies, 2

(0.14%) were confirmed to have hearing loss. This study reveals, the prevalence of congenital hearing loss in our population is

1.42 per 1000 babies.

Prevalence and referral rates in neonatal hearing screening program using two step hearing screening protocol in Chennai – A prospective study

S.S. Vignesh V. Jaya B.I. Sasireka Kamala Sarathy M. Vanthana

Intnl J. Ped Otolaryngol. October 2015Volume 79, Issue 10, Pages 1745–1747

1405 Babies 70% Well

30% High Risk

DPOAE

Screen

311 Fail 22.13%

31 Fail 10% group 2.2% total

ABR

Screen

2 Confirmed

6.45% group .14% total

17

Newborn

In-Patient

Up to 2

Screens

Pass

Out

Out Patient

Diagnostic

Fail WBN

Outpatient

Screen

Normal

Out

NICU fail Loss Intervention

Pass

Out

California Screening Flow Chart

18

Protocols

• Two Screenings at birth will reduce referral rate.

• In US, most babies go home early (1 day) increasing the

middle ear false positives seen with OAE.

• Some states require an “outpatient” rescreen to reduce

referral rates. This may be only for well babies.

• Over-referral is a major issue for audiology clinics in US.

19

Protocol Choices/Technology

OTOACOUSTIC EMISSIONS

+Lower initial cost for supplies +Less patient preparation (time) -Higher refer rate (up to 20%) -Intolerant to Ambient Noise -Insensitive to Neural Dysfunction

ELECROPHYSIOLOGY ABR/ASSR

-Higher disposable supply costs -Electrode application time +Lower refer rate <5% +Less sensitive to acoustic noise +Will detect auditory nerve & brainstem dysfunction

20

EHDI in the United States

• The 2011 statistics indicate that 65.1 percent of the children

referred for additional testing will have an Audiological

Diagnostic Evaluation.

• Of those, 71.8 percent were identified by 3 months of age.

So there still a significant number of infants with hearing

loss not diagnosed by 3 months of age.

• Loss to follow-up and delays in fitting of amplification are

still significant problems in the US.

21

Problems

• Loss to follow-up, insufficient tracking.

• SIGNIFICANT delays for diagnostic appointments and need

for multiple test dates.

• Delays in funding for hearing aids.

• Lack of standards for Early Intervention.

22

Problems/Solutions? Late identification of AN in Well Babies

• Education of professionals on short-comings of some

protocols.

• Ensure that infants who refer from ABR are not rescreened

with otoacoustic emissions.

• Reduce the time and cost of Electrophysiologic Screening.

• Expand Genetic Testing protocols

23

Fitting of Amplification by six months:

“we're happy to have financial

support from some of the hearing

aid manufacturers, such as Oticon

and Phonak, Starkey, and Widex.

Their donations and hearing aid

loaner banks make a world of

difference with regard to fitting

babies with hearing aids—but that

only happens when the centers

taking care of the babies know these

opportunities are available.”

Christie Yoshinaga-Itano:

24

Newborn

In-Patient

Up to 2

Screens

Pass

Out

Out Patient Diagnostic

Fail WBN

Outpatient

Screen

Normal

Out

NICU fail Loss Intervention

Pass

Out

Biggest Problem Area in US

25

Problems Delays at Diagnostic Stage

• Long test times prevent diagnostic assessment

from being completed in one session.

• Multiple sessions reduce the confidence of family

and increase stress.

• Added sessions increase the risk of loss to follow

up and missed appointments.

26

Problems Delays at Diagnostic Stage

• Cost to the families, health care systems and

audiology providers increases with multiple sessions.

• Systems pay by test and not by time so clinics lose $.

• Long appointment times and need for specialized

skills limits the number of clinics that perform

diagnostic evaluations.

27

Which Problems would be reduced by faster test times?

• Long test times prevent diagnostic assessment

from being completed in one session.

• Multiple sessions reduce the confidence of family

and increase stress.

• Added sessions increase the risk of loss to follow

up and missed appointments.

28

Which Problems would be reduced by faster test times?

• Cost to the families, health care systems and

audiology providers increases with multiple sessions.

• Systems pay by test and not by time so clinics lose $.

• Long appointment times and need for specialized

skills limits the number of clinics that perform Dx

evals

29

Problems Delays at Diagnostic Stage

• Leads to delays in

fitting of amplification

and enrollment into

early intervention!!

30

Solutions? Delays at Diagnostic Stage

Reduce false positive referrals.

Better tracking and follow-up procedures.

Parent Education.

Reduce diagnostic test time. (One session in < 2 hours).

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20

60

80

100

120

0

40

dB

Hea

rin

g L

eve

l (A

NS

I, 1

99

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Frequency (Hz)

250 500 1000 2000 4000 8000

O

O

O O

X X X X

Audiology Diagnostics Following NBHS

1. Electrophysiology to predict

thresholds for air and BONE

Conduction

2. Diagnostic OAE

3. Immittance (acoustic reflex)

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35

500 Hz Tone Bursts

Milliseconds

0 5 10 15 20 25 30

dB nHL

0.1 V

Fsp Noise Sweeps

7.25 33.7 1536

3.27 32.5 4352

0.93 28.3 3072

36

2000 Hz

Milliseconds

0 5 10 15 20 25 30

70

dB nHL

0.2 V

Fsp Noise Sweeps

7.25 33.7 1536

3.27 32.5 4352

3.19 23.8 2560

0.93 28.3 3072

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38

20

60

80

100

120

0

40

dB

Hea

rin

g L

eve

l (A

NS

I, 1

99

6)

Frequency (Hz)

250 500 1000 2000 4000 8000

O

O

O O

X X X X

Audiology Diagnostics Following NBHS

PREDICTED THRESHOLDS

• Up to 4 Frequencies

• Both ears

• Bone conduction

HOW CAN THIS BE

ACCOMPLISHED MORE

QUICKLY??

39

Ferm, Lightfoot & Stevens International Journal of Audiology 2013;

1000 Hz NB CE-chirp 1000 Hz Tone Pip 4000 Hz NB CE-chirp 4000 Hz Tone Pip

40 30 20 10 0

45 35 25 15

NB CE-Chirps Deliver More Amplitude than Tone Pips

40

Simultaneous multi-frequency ASSR-testing Band-limited Chirps

500 Hz 1,000 Hz 2,000 Hz 4,000 Hz

500 Hz - one octave

1,000 Hz - one octave

2,000 Hz – one octave

4,000 Hz - one octave

41

“The present study supports the findings of other groups, showing that multiple-frequency 40Hz ASSRs accurately predict behavioural audiograms in adults with normal hearing and moderate sensorineural hearing loss.” “The use of optimized octave-band chirp stimuli and a semi-automatic adaptive recording algorithm reduces the total test duration considerably.“ The average test time (threshold, 4 frequencies, both ears) reported: 18.6 minutes Note: These patients were sedated

42

ECLIPSE ASSR – examples of test time.

(4 freq. down to threshold in both ears)

Deborah Carlson (in progress)*: Adults NH 40Hz (n=50): 30 min Adults NH 90Hz (n=50): 30 min Rodrigues and Lewis (in press)*: Natural sleeping NH neonates (n=30): 21,1 min Rebiero and Chapchap 2011: Natural sleeping term babies (n=28): 51 min Natural sleeping preterm babies (n=17): 33 min *) Also sources of nHL-eHL corrections: Similar for infants and adults (90Hz) and approx: 500Hz: 25dB 1kHz: 15dB 2kHz: 10dB 4kHz: 5dB

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2000 Hz 4000 Hz

F. Venail et al. Narrow band CE-Chirps evoked ASSR in Children International Journal of Audiology 2014; Early Online: 1–8

French Study Shows Excellent Prediction of Infant/Toddler Thresholds Using Enhanced ASSR Detection & NB CE-Chirps

44

F. Venail et al. Narrow band CE-Chirps evoked ASSR in Children International Journal of Audiology 2014; Early Online: 1–8

500 Hz 1000 Hz

French Study Average time for 8 Frequencies with ASSR is 22 minutes;

for click ABR - 13 minutes

45

46

QUESTIONS?

2013 JCIH POSITION STATEMENT UPDATE TO 2007 PEDIATRICS Volume 131, Number 4, April 1, 2013

Pediatrics

Principles and Guidelines for Early Intervention After

Confirmation That a Child Is Deaf or Hard of Hearing

48

Goal 4: All Children Who Are D/HH With Additional Disabilities and Their Families

Have Access to Specialists Who Have the Professional Qualifications and Specialized

Knowledge and Skills to Support and Promote Optimal Developmental Outcomes

Goal 5: All Children Who Are D/HH and Their Families From Culturally Diverse

Backgrounds and/or From Non–English-Speaking Homes Have Access to Culturally

Competent Services With Provision of the Same Quality and Quantity of Information

Given to Families From the Majority Culture

Goal 3a: Intervention Services to Teach ASL Will Be Provided by Professionals Who Have

Native or Fluent Skills and Are Trained to Teach Parents/Families and Young Children

Goal 3: All Children Who Are D/HH From Birth to 3 Years of Age and Their Families

Have EI Providers Who Have the Professional Qualifications and Core Knowledge and

Skills to Optimize the Child’s Development and Child/Family Well-being

Goal 3b: Intervention Services to Develop Listening and Spoken Language Will Be

Provided by Professionals Who Have Specialized Skills and Knowledge

Goal 2: All Children Who Are D/HH and Their Families Experience Timely Access to Service

Coordinators Who Have Specialized Knowledge and Skills Related to Working With Individuals

Who Are D/HH

49

Goal 6: All Children Who Are D/HH Should Have Their Progress Monitored

Every 6 Months From Birth to 36 Months of Age, Through a Protocol That

Includes the Use of Standardized, Norm-Referenced Developmental

Evaluations, for Language (Spoken and/or Signed), the Modality of

Communication (Auditory, Visual, and/or Augmentative), Social-Emotional,

Cognitive, and Fine and Gross Motor Skills

Goal 7: All Children Who Are Identified With Hearing Loss of Any Degree,

Including Those With Unilateral or Slight Hearing Loss, Those With

Auditory Neural Hearing Loss (Auditory Neuropathy), and Those With

Progressive or Fluctuating Hearing Loss, Receive Appropriate Monitoring

and Immediate Follow-up Intervention Services Where Appropriate

Goal 8: Families Will Be Active Participants in the Development and

Implementation of EHDI Systems at the State/Territory and Local Levels

50

Goal 9: All Families Will Have Access to Other Families Who Have

Children Who Are D/HH and Who Are Appropriately Trained to Provide

Culturally and Linguistically Sensitive Support, Mentorship, and

Guidance

Goal 10: Individuals Who Are D/HH Will Be Active Participants in the

Development and Implementation of EHDI Systems at the National,

State/Territory, and Local Levels; Their Participation Will Be an

Expected and Integral Component of the EHDI Systems

Goal 11: All Children Who Are D/HH and Their Families Have Access to

Support, Mentorship, and Guidance From Individuals Who Are D/HH

Goal 12: As Best Practices Are Increasingly Identified and Implemented, All

Children Who Are D/HH and Their Families Will Be Ensured of Fidelity in

the Implementation of the Intervention They Receive

51